Document Detail

Acute lymphoid leukemia associated with Maffucci's syndrome.
MedLine Citation:
PMID:  8214367     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Maffucci's syndrome is a nonhereditary congenital disorder associated with multiple enchondromas, soft tissue hemangiomas, or lymphangiomas. It carries an associated high risk of the development of malignant neoplasms, particularly sarcomatous transformation of an enchondroma, as well as other malignant mesodermal and nonmesodermal neoplasms. Hematopoietic malignancies arising in Maffucci's syndrome are exceedingly rare. We report the case of a 14-year-old girl with Maffucci's syndrome who developed acute lymphoid leukemia. PATIENTS AND METHODS: The patient presented at 18 months of age with enchondromatosis. Maffucci's syndrome was established at 10 years of age after the appearance of multiple hemangiomas. RESULTS: At 14 years of age the patient developed fatigue, frequent nosebleeds, easy bruising, and weight loss, with circulating blasts in the peripheral blood. Bone marrow examination showed replacement of marrow spaces with leukemic blasts. Immunohistochemical and flow cytometric findings were consistent with a diagnosis of acute lymphoblastic leukemia with myeloid antigen expression. CONCLUSIONS: The occurrence of acute leukemia in a patient with Maffucci's syndrome may represent predisposition to yet another malignancy and reflect further expression of a generalized mesodermal dysplasia in these patients. It also emphasizes the need for aggressive surveillance in patients with Maffucci's syndrome.
J T Rector; C L Gray; R W Sharpe; F W Hall; W Thomas; W Jones
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The American journal of pediatric hematology/oncology     Volume:  15     ISSN:  0192-8562     ISO Abbreviation:  Am J Pediatr Hematol Oncol     Publication Date:  1993 Nov 
Date Detail:
Created Date:  1993-11-19     Completed Date:  1993-11-19     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7908071     Medline TA:  Am J Pediatr Hematol Oncol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  427-9     Citation Subset:  IM    
Department of Laboratory Medicine, Naval Hospital, San Diego, California 92134-5000.
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MeSH Terms
Antigens, CD / blood
Bone Marrow / pathology
Enchondromatosis / complications*,  pathology,  radiography
Hemangioma / complications,  pathology
Lymphocytes / immunology
Precursor Cell Lymphoblastic Leukemia-Lymphoma / blood,  complications*,  immunology,  pathology
Skin Neoplasms / complications,  pathology
Reg. No./Substance:
0/Antigens, CD

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